门-窦血管病——门静脉高压症的一种新型疾病谱

李玉林; 符艳秋; 周丽诗; 李雨芙; 羊东晔

doi:10.3969/j.issn.1001-5256.2022.12.032

门-窦血管病——门静脉高压症的一种新型疾病谱

DOI: 10.3969/j.issn.1001-5256.2022.12.032

1.
深圳大学医学部，广东深圳 518071
2.
香港大学深圳医院消化及肝脏科，广东深圳 518053

基金项目:

2018年深圳市科技计划基础研究(学科布局)项目 (JCY20180508153013853)

利益冲突声明：所有作者均声明不存在利益冲突。

作者贡献声明：李玉林负责检索文献及撰写文章；符艳秋、周丽诗、李雨芙负责分析和总结文献；羊东晔负责提出思路，指导写作及修改文章。

详细信息

通信作者:
羊东晔，yangdy@hku-szh.org

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出版历程
- 收稿日期: 2022-05-15
- 录用日期: 2022-06-20
- 出版日期: 2022-12-20

Porto-sinusoidal vascular disease——A novel entity of portal hypertension

Yulin LI¹,
Yanqiu FU¹,
Lishi ZHOU²,
Yufu LI¹,
Dongye YANG^{2
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1.
Shenzhen University Health Science Center, Shenzhen 518071, China
2.
Division of Gastroenterology & Hepatology, The University of Hong Kong-Shenzhen Hospital, Shenzhen 518053, China

Research funding:

Shenzhen Science and Technology Innovation Commission Fundamental Research Key Projects of 2018 (JCY20180508153013853)

More Information

Corresponding author: YANG Dongye, yangdy@hku-szh.org (ORCID: 0000-0003-3558-8871)

摘要

摘要: 门-窦血管病(PSVD)是一种以门静脉高压症为主要临床特征，但无肝硬化，或无肝硬化相关危险因素，且影像学检查门静脉、肝静脉及下腔静脉通畅，需要活检病理诊断的一种疾病。该疾病在东西方的发病率存在很大差异；已知与自身免疫、血液病及血栓前疾病、感染、毒物或药物暴露、遗传、代谢相关，但确切的病因仍然未知。该疾病有三种典型组织病理学改变，分别是闭塞性门静脉病、结节性再生增生、不完全性间隔纤维化。PSVD早期临床表现没有特异性，晚期与肝硬化相似，影像上主要表现为门静脉高压相关并发症的征象，但肝脏硬度正常或稍增加，诊断需要肝活检；治疗上参照肝硬化的标准；该疾病预后明显优于肝硬化。该综述回顾性总结PSVD的流行病学、发病机制、临床诊疗等，以期提高临床医生对该病的认识。
- 高血压, 门静脉 /
- 诊断 /
- 治疗学
Abstract: Porto-sinusoidal vascular disease (PSVD) is an entity characterized with portal hypertension (PH) in the absence of cirrhosis, the related risk factors, and imaging evidence of obstructed portal vein, hepatic vein and inferior vena cava. Its prevalence varies significantly between East and West countries. Until now, the etiologies have been classified as autoimmune, hematologic, and prethrombotic conditions, infections, toxins or drugs, and genetic or metabolic disorders. However, the definite cause remains unknown. Diagnosis is based on three histopathological features: obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal fibrosis. The clinical manifestations of early PSVD are nonspecific, whereas those at a late stage are similar to cirrhosis. The imaging detection mainly reveals the PH signs and complications, but the liver stiffness is normal or slightly increased, necessitating a liver biopsy for PVSD diagnosis. PSVD treatment is similar to liver cirrhosis; however, the prognosis is better. In order to gain a thorough understanding of PSVD, the epidemiology, pathogenesis, clinical diagnosis, and treatment are discussed in this article.
- Hypertension, Portal /
- Diagnosis /
- Therapeutics

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表 1 PSVD组织学特征

Table 1. Histological features of porto-sinusoidal vascular disease

直接证据	间接证据
闭塞性门静脉病变结节性再生增生不完全性间隔纤维化	肝窦扩张，巨窦窦周纤维化中央静脉异常门静脉疝，门静脉周围异常血管，门静脉残余，门静脉多样性等
注：(1)组织学特征满足直接证据之一者就可诊断PSVD；(2)组织学特征满足一项或多项间接证据需怀疑PSVD。

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表 2 PSVD诊断标准

Table 2. Diagnostic criteria for porto-sinusoidal vascular disease

肝活检(长度≥20 mm或≥10门静脉束或病理学家认为足够)排除肝硬化且排除其他原因PH

(1)满足任意一条典型临床表现：①胃食管或异位静脉曲张；②门静脉高压性出血；③影像学可见侧支循环形成
(2)满足任意一条典型组织学特征：①闭塞性门静脉病；②结节性再生增生；③不完全性间隔纤维化
(3)任意一条非典型临床表现：①脾肿大；②腹水；③血小板减少+任意一条非典型组织学特征：a.门静脉通道异常(动脉增殖、扩张等)；b.结构异常(门静脉束和中央静脉分布不规则)；c.肝窦扩张；d.轻度窦周纤维化

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